Assessment of Mental Retardation

7/28/2019 Assessment of Mental Retardation

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ASSESSMENT OF MENTAL

RETARDATIONPresenter: Dr. Pavan Kumar Kadiyala

Chairperson: Dr. V.K. Bhat


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INTRODUCTION

Mental retardation (ICD 10) arrested or incomplete development of the mind,

impairment in overall level of intelligence

impairment of cognitive,

language,

motor & social skills

during developmental period

Adaptive behaviour impaired always

DSM IV TR

Significant subaverage general intellectual functioning significant limitations in adaptive functioning

onset before 18 years of age


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IMPORTANCE

At least one-third of children attending

Child Psychiatry OPDs or Child

Guidance Clinics have MR

MR causes more suffering than otherdisabilities

Care requires both scientific and

humanistic outlook


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INTELLIGENCE

Global ability to reason,

plan,

solve problems,

think abstractly,

comprehend complex ideas,

learn quickly and

from experience to adapt

effectively to the environment .(Weschler)


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Milestones of development

AGE MOTOR SOCIAL LANGUAGE COGNITIVE

2 - 4months Holds headsteadily while

prone

Social smile Cooing Visual trackingAuditory

localization

6 -8

months

Sits without

support

Stranger

anxiety

Babbling,

Takes objects

on request

Reaching for

objects

10-12 months stands without

support,

Simple social

imitation

Gives objects

on request

vocal imitates

Object

permanence

13-15 months Walks without

support

Separation

anxiety

First words Pointing to

objects

2 years Runs well Imitate

mannerisms


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EPIDEMIOLOGY

Prevalence of MR- 1-3% of population.

Highest incidence in school age children

with peak ages 10-14 yrs.

M:F=1.5:1

Developing> Developed countries.

Iodine deficiency MC preventable cause worldwide.

Regions with high consanguineous marriages & exposure toheavy metals and toxins.

.

Prevalence in India:

As per NIMHANS- 0.2% for mild MR; 0.5% for severe MR.

1/3rd of children attending child psychiatry OPD have MR. As per Inst. of Child Development, New Delhi, 3 out of 100

children in the country have MR


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ICD-10 F70-F79

F70 Mild Mental Retardation F71 Moderate Mental Retardation

F72 Severe Mental Retardation

F73 Profound Mental Retardation

F78 Other Mental Retardation

F79 Unspecified Mental Retardation

4th character to specify ass behavior impairment.

F7x.0 No or minimal impairment of behavior

F7x.1 significant impairment requiring attention or treatment F7x.8 other impairments of behaviour

F 7x.9 without mention of impairment of behaviour


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DSM IV TR CRITERIA FOR MR

A . Significantly subaverage intellectualfunctioning: an IQ of approx 70 or below onindividually administered IQ test

B . Concurrent deficits or impairments inpresent adaptive functioning in at least 2 offollowing areas: communication , self care,home living , social / interpersonal skills, use

of community resources , self direction ,functional academic skills , work, leisure ,health and safety.

C . The onset is before 18 years


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Degree of Severity DSM IV TR

317 Mild MR

318.0 Moderate MR

318.1 Severe MR

318.2 Profound MR

319 MR severity unspecified ; strong

presumption of MR but persons

intelligence is undetectable by standard

tests


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Classification

CLASSIFICATION ICD 10IQ LEVEL

DSM IV TRIQ LEVEL

MILD MR 50-69 50-55 to approx 70

MODEARATE MR 35-49 35-40 to 50-55

SEVERE MR 20-34 20-25 to 35-40

PROFOUND MR


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DEGREE OF MR with IQ range as per ICD 10

ADULT ATTAINMENT

++ means definitely attainable: + means

attainable: +/- means sometimes

attainable

Mild (50-79) Literacy ++

Self-help skills++

Good speech ++

Semi-skilled work +

Moderate (35-49) Literacy +

Self-help skills ++

Domestic speech+

Unskilled work with or without supervision +

Severe (20-34) Assisted self-help skills+

Minimum speech+

Assisted household chores +

Profound (


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ETIOLOGIC CLASSIFICATION

OF MRPrenatal perinatal postnatal

EmbryonicChromosomal

Single gene disorders

Dysmorphic syndromes

MaternalInfections

Deficiencies

Maternal diseases

Pregnancy related

III trimester

complications

Prematurity & low birthweight

HIE (birth asphyxia)

CNS infections

Chronic leadpoisoning

Head injury

Malnutrition

understimulation


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Chromosomal disorders

Down syndrome,

Klinefelter syndrome,

Turner syndrome,

Cri-du-chat syndrome,

Prader Willi syndrome,

Angelman syndrome,

William syndrome


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Inborn errors of metabolism:

Galactosemia,

Phenylketonuria,

Mucopolysaccharidoses,

Tay- Sachs disease,

Lesch-Nyhan syndrome,

Hypothyroidism,


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Neuro-cutaneous: Tuberous sclerosis,and neurofibromatosis

Brain malformations such as autosomal

recessive primary microcephaly,hydrocephalus

Others: fragile X syndrome, Rett

syndrome,Laurence Moon Bardet Biedlsyndrome, Smith-Lemli-Opitz syndrome,

Coffin Lowry syndrome


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Other conditions of uncertain

genetic origin Rubinstein Taybi syndrome

De Lange syndrome


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Adverse maternal /environmental

influences Deficiencies: iodine deficiency,

folate deficiency

Severe malnutrition in pregnancy

Usingsubstances:

alcohol (maternal alcohol syndrome),

nicotine, and

cocaine during early pregnancy


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Adverse maternal /environmental

influences

Exposureto other harmful chemicals:

pollutants,

heavy metals,

abortifacients, and

teratogenic medications such as

thalidomide,

phenytoin and

warfarin sodium in early pregnancy


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Maternal infections: rubella,

Syphillis,

Toxoplasmosis,

Cytomegalovirus,

Herpes and

HIV

Others: excessive exposure to radiation, Rh

iso-immunization


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Perinatal

Third trimester

Labour

Neonatal


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Third trimester

Complications of pregnancy:Ecclampsia

Maternal Diseases: cardiac, renal,

diabetes Placental dysfunction /deprivation of

supply


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Labour

Severe prematurity,

Very low birth weight,

Hypoxic ischemic encephalopathy

(birth asphyxia),

Difficult and/or complicated delivery,

Birth trauma


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Neonatal

Septicemia,

severe jaundice,

hypoglycemia


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Postnatal

Brain infections: tuberculosis,

Japanese encephalitis, and

bacterial meningo-encephalitis

Head injury

Chronic lead exposure

Severe and prolonged malnutrition

Gross understimulation and experiential

deprivation

C bidit


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Comorbidity Upto 2/3rd with MR have comorbid mental disorders.

Full range of psychiatric disorders.

Prevalence of psychopathology correlated to severity of MR.

Disruptive & conduct-disorder behavior MC in mild MR.

Severely retarded group associated with autistic disorder and PDD

Others-

Mood disorders (50%)

Schizophrenia(3%)

ADHD (7-15%)

Conduct disorder ,PDD

Behavior disorder

Non-syndromal: restlessness, self-injurious behaviors, aggression,stereotypies, impulsivity, pica


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standardized instruments

To screen for psychiatric and behavioraldisorders

Psychiatric Assessment Schedule for

Adults with Developmental Disability(PAS-ADD),

Reiss Screen for Maladaptive Behavior,

Psychopathology Inventory For MentallyRetarded Adults (PIMRA),

Developmental Behavior Checklist (DBC

MEDICAL DISORDERS


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MEDICAL DISORDERS

Seizure disorder

Cerebral palsy Visual or Hearing impairment

Congenital heart disease

Cleft lip and cleft palate

Orthopedic handicaps (CTEV, CDH) Vitamin and mineral deficiencies

Recurrent infections

Psychosocial features:

Negative self-image

Poor self-esteem social withdrawal


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Dimensions of clinical evaluation

in MR Detailed history

Thorough physical examination

Psychological testing

Measure of adaptive function

Family & psychosocial features should be

assessed

Physical investigations

Comprehensive diagnosis

Clinical questions to guide


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Clinical questions to guide

evaluation

What precipitated the consultation? Is there significant developmental delay?

Is it global or restricted?

How severe is the delay?

What is the cause/s? is there a treatable cause?

What is the recurrence risk?

Are there associated medical, behavioral or

psychiatric problems? How much do the parents know about the

condition?

What are the difficulties facedby them?


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CLINICAL PRESENTATION

delayed milestones of development,

poor ability to learn new things,

poor speech and comprehension,

poor self-help skills, and poor schoolperformance,

poor memory are the common presentingcommon complaints.

behavior problems - restlessness, poorconcentration, impulsivity, self-injurious behavior,or

sleep / appetite disturbances


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History taking

Complaints

Family history

Personal history

Medical history

Psychiatric history

Treatment history

Current developmental attainments


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Physical examination:

Head-to-toe examination of all the organsystems

Special attention should be paid toneurological examination.

Document any minor congenital anomalies(MCAs) (Smiths Recognizable Patterns of

Human Malformation) Presence of 4 or more MCAs is a pointer to

a prenatal etiology

MINOR CONGENITAL


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MINOR CONGENITAL

ANOMOLIES( MCAs)

Height: short stature, tall stature,increased arm span, gigantism

Weight: obesity, emaciation

Facial appearance: typical facies(mongoloid, coarse), elongated,triangular

Head circumference: microcephaly,macrocephaly

Shape of skull: brachycephaly,scaphocephaly, trigonocephaly,oxycephaly, plagiocephaly


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contd

Eyes: deeply set, proptosis,microphthalmia, upslanting /

downslanting eyes, hypertelorism,

epicanthal folds, strabismus, nystagmus,ptosis, bushy eyebrows, K-F ring,

cataracts, coloboma of iris, blue sclera,

telangiectasia


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contd

Hair: hirsutism, light colored, doublewhorl on scalp, easily breakable,

Neck: short, webbed, torticollis

Hands: simian crease, Sidney line, spadeshaped

Fingers: clinodactyly, camptodacyly,

arachnodactyly, short little finger,syndactyly, polydactyly, broad thumb


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contd

Chest: pectus excavatum, pectus carinatum, nippleanomalies, gynaecomastia

Abdomen: protuberant, umbilical hernia, hepato-splenomegaly, inguinal hernia

Spine: kyphosis, scoliosis, spina bifida

External genitalia: hypogenitalism, macro-orchidism,undescended testis, ambiguous genitalia, hypospadias, absent

secondary sexual charactersitcs, shawl scrotum

Feet: pes planus, pes cavus, valgus / varus anomaly, broadhallux, increased distance between 1st & 2nd toe


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Syndrome seen in INDIA Key features

Down syndrome Typical facies, short stature, medial slanting of eyes, clinodactyly,

simian crease, cup-shape ears

Fragile X syndrome Elongated, triangular face, protruding /prominent ears, macro-orchidism in post-pubertal boys

Rett syndrome Normal development till around 1 year of age in a girl child followed

by plateauing and regression, loss of hand functions, mid-line hand

stereotypies

De Lange syndrome Hirsutism, long eye-lashes, synophrys, bushy eye brows,

microcephaly,

Prader Willi syndrome Obesity, hypogenitalism,

Tuberous sclerosis Sebaceous adenomas, ash-leaf spots, shagreen patches, seizures

Congenital hypothyroidism Lethargy, growth failure, coarse and dry skin, constipation, feeding

problems, protuberant abdomen, bradycardia

Mucopolysaccharidoses Typical facies, coarse skin, skeletal anomalies, macrocephaly

Homocystinuria

Phenylketonuria

Marfanoid features,

Light colored hair, abnormal smell of urine, microcephaly, seizures


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Clinical interview in MR

Setting for interview:Toys, Books, Pictures, Paper, pencil

Couch, child friendly furniture

Process of Interviewing

Building rapport

Make the kid and parents comfortable

Verbal interviewing: depends on language development andconversational skills:

Simple, structured, and brief;

Use clear & concrete questions

Avoid leading questions

Use parents when necessary for interviewing


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Clinical observation

Basics: Vision, hearing, locomotion, physical health Attention, concentration

Response to interview situation

Sociability

Motor Activity level Impulse control

Speech, language & communication

Mood

Play behavior

Other inappropriate behaviors: (stereotypies, Self-Injurious Behavior),

Impressions on current developmental attainment

INVESTIGATIONS


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INVESTIGATIONSUrine screen for abnormal metabolites Phenyketonuria, homocysteinuria, galactosemia, MPS

Thyroid function test Hypothyroidism

Advanced metabolic tests (Gas chromatographic Mass

Spectroscopyc (GCMS), tandem mass spectroscopy (TMS)

Wide range of neuro-metaboloic disorders such as fatty acid

oxidation disorders, aminiacidopathies, urea cycle disorders

and organic acidurias

Enzyme studies Tay-Sach disease, meatachromatic leukodystrophy

Karyotyping Down syndrome, other chromosomal disorders

FISH Prader Willi syndrome, William syndrome, Sub-telomeric

deletions

Molecular genetics Fragile X syndrome (FMR1 mutation), Rett syndrome

(MECP2 mutation),


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Investigations contdBrain imaging Tuberous sclerosis, lissencepahly,

EEG Epileptic encephalopathies such as West syndrome

Hearing evaluation (BAER) Sensory-neural hearing impairment

Visual evaluation Wilson disease, cataract, Optic atrophy, cortical

blindness, refractive error

Blood group of child and parents Rh iso-immunization

Immunologic tests (Ig M antibodies) TORCH infections


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Psychological testing

Commonly used tests in India are Vineland Social Maturity Scale (VSMS),

Binet Kamat Test (BKT),

Malins Intelligence Scale for Indian Children

(MISIC),

WISC, and

Bhatia Battery.

An Indian adaptation of Baileys Scale for infantsis also available (DASI).

Checklists such as Portage checklist, BASIC MRfrom NIMH, Secunderabad, DDVP, Trivandrum

modified Rutters multi-axial


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modified Rutter s multi axial

system for comprehensive

diagnosis I: Presence and degree of MR (mild MR)

II: Etiologic / syndromal diagnosis (fragile Xsyndrome)

III: Associated medical problems (epilepsy)

IV: Associated psychiatric problems (ADHD)

V: Family & psycho-social axis (poorawareness, high stress levels, overexpectation)


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DIFFERENTIAL DIAGNOSIS

cerebral palsy without MR, pervasive developmental disorder without

MR,

specific learning disability or

specific delays in development of scholasticskills (dyslexias),

specific delay of speech and languagedevelopment,

severe emotional disorder and visual and hearing impairment may be

erroneously labeled as MR


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MANAGEMENT OF MR

Parent Counseling Treatment of the underlying disorder wherever possible

Early intervention in children who are at risk and those who already havedevelopmental delay

Management comorbid psychiatric and medical problems

Individualized training program for the child based on assets and liabilities

in the child, family and environment Parent training for home-based management

Referrals for special education, physio-occupational therapy, speechtherapy, vocational training, and parent organizations

Discussion about parental concerns such as social security, guardianship,menarche, marriage, etc and providing appropriate guidance

Helping parents to access social welfare benefits etc Checking about the need for genetic counseling and offering appropriate

help

M di l i i i MR


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Medical interventions in MR

Diagnosis and treatment of treatableunderlying disorders

E.g., Hypothyroidism, PKU

Diagnosis and treatment of comorbidmedical & psychiatric problems

E.g., Epilepsy, hearing impairment, ADHD,Undernutrition, feeding and sleeping

problems Genetic counseling

Management of comorbid


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Management of comorbidpsychiatric and behavioral

disorders Persons with MR respond to variouspsychotropic medications in ways similar tothe typically developing population

efficacy of risperidone in aggression andstereotypies,

clonidine in hyperactivity and impulsivity(especially in the presence of seizures as

clonidine has no effect on seizurethreshold,and

SSRIs in dysphoria, SIB and stereotypies

Psycho-social management of


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Psycho social management ofMR

Individual interventions

Behavior modification techniques for

building new skills

Behavior modification techniques foreliminating odd or problem behaviors

Behavior modification


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Behavior modificationtechniques for building new

skills Technique

Goal specification

Task analysis

Rewarding Modeling

Shaping

Chaining

Back chaining Forward chaining

Prompting



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techniques for eliminating odd

or problem behaviors Disregarding

Ignoring

Redirecting

Limit-setting Blocking

Gradual guidance

Time-out (from positive reinforcement)

Differential reinforcement of other behavior Over-correction

Response cost

Family-focused intervention in


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Family focused intervention inMR

Parent counseling Oppositional defiant behaviors, tantrums,

and other disruptive behaviors in these

children mainly as a learnt behavior inresponse to faulty parent child

relationships and child-rearing practices.

effectively tackled through parentcounseling, behavior modification, and

parent management training.

PREVENTION OF MR


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PREVENTION OF MR

PRIMARY PREVENTION

(preventing the occurrence of retardation)

Health promotion

Specific protection


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contd

SECONDARY PREVENTION(halting disease progression) Early diagnosis and treatment

Neonatal screening for treatable disorders (hypothyroidism,phenynlketonuria, galactosemia, homocysteinuria, congenitalhydrocephalus)

Intervention with at risk babies

Early detection and intervention of developmental delay TERTIARY PREVENTION

(preventing complications and maximization of functions)

Disability limitation and rehabilitation

Stimulation, training, and education, and vocational opportunities

Mainstreaming / integration Support for families

Parental self-help groups

SOCIAL AND COMMUNITY


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SOCIAL AND COMMUNITYLEVEL INTERVENTIONS IN MR

Persons with Disabilities Act National Trust for welfare of persons with

autism, cerebral palsy, mental retardationand multiple disabilitites Act 1999

National Policy on Disability.

National Institute for the MentallyHandicapped (NIMH, Secunderabad):

Sarva Shiksha Abhiyan Respite and residential care facilities

NGO Sector


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THANK U

Assessment of Mental Retardation

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